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Biomedical subjects

J Bodensteiner

Publications and source records attributed to J Bodensteiner.

32 records · Page 2Linked to original sources
PubMed1990

Colpocephaly: pitfalls in the diagnosis of a pathologic entity utilizing neuroimaging techniques.

Colpocephaly has been described as the persistence of the fetal configuration of the lateral ventricles. The pathologic picture is characterized by multiple features of disturbed or arrested development of the brain, which results in diminished thickness of the cerebral white matter in the posterior portion of the centrum semiovale, giving rise to large occipital horns of the lateral ventricles. This ventricular configuration allows the clinician to suspect the presence of this developmental disturbance utilizing computed tomographic images. In this paper, we present a case that demonstrates that not every patient with enlargement of the posterior horns of the lateral ventricles has the underlying developmental abnormalities that constitute colpocephaly as described by Yakovlev and Wadsworth. Furthermore, we point out the difficulties that can arise in the attempt to make a diagnosis of a pathologically defined condition on the basis of neuroimaging results alone.

Cerebral Palsy↗
PubMed1989

X-linked olivopontocerebellar atrophy.

We present a kindred with a relatively pure cerebellar degeneration that demonstrates X-linked recessive inheritance. The unique clinical picture of affected patients in our kindred is characterized by an infantile onset of ataxia; very slow rate of progression; normal strength, reflexes, and sensation; and cerebellar degeneration with involvement of the olive and pons demonstrated by neuroimaging techniques. The distinction between this and other reported olivopontocerebellar degenerations is made on the basis of the clinical features and mode of inheritance. It is not clear if the distinct clinical pattern in this kindred represents variable expression of a previously reported condition, allelic variance of previously reported kindreds, or a separate clinical entity. Molecular analysis, currently underway, may help settle the issue.

Adult↗
PubMed1988

Congenital myopathies.

The evaluation of the "floppy infant" is a common problem for neurologists who deal with infants. One major category of diseases to consider in the diagnosis of hypotonia in the infant is the congenital myopathies. The congenital myopathies are a group of nonprogressive primary diseases of muscle that present in infancy with hypotonia and weakness and that are subdivided on the basis of the morphologic characteristics seen on histochemical study of the muscle. These diseases share many clinical features despite demonstrating a wide range of variation. Through the study of the various congenital myopathies, considerable knowledge of the structural components of the muscle fiber and their function has been gained. Despite this, our understanding of these conditions is incomplete at this time.

Humans↗
PubMed1986

Progressive ascending paralysis following administration of intrathecal and intravenous cytosine arabinoside. A Pediatric Oncology Group study.

Two childhood acute myelogenous leukemia (AML) patients receiving intrathecal (IT) and intravenous (IV) cytosine arabinoside (Ara-C) developed progressive ascending paralysis, resulting in death in one patient. Necropsy findings on this patient included spinal cord demyelination characteristic of Ara-C neurotoxicity. An unusual aspect of these two cases was the delay between cessation of IT therapy and the onset of neurologic symptoms. These patients received relatively low total doses of IT Ara-C and standard doses of IV Ara-C. Previous studies have shown that Ara-C equilibrates readily between serum and cerebrospinal fluid; this implies that total IV and IT doses of Ara-C may be additive in relation to development of neurotoxicity. For these reasons, use of IV and IT Ara-C in childhood AML must be approached with greater caution, especially if neurologic abnormalities develop during or after therapy.

Acute Disease↗
PubMed1986

Dominantly inherited craniodiaphyseal dysplasia: a new craniotubular dysplasia.

We describe a mother and her male infant affected with a craniotubular dysplasia characterized by severe craniofacial hyperostosis and sclerosis with obliteration of paranasal sinuses and foramina of the skull base. Subsequent severe bilateral hearing loss and facial diplegia with relative sparing of the optic nerves were noted. The long bones show extreme asymmetric hyperostosis and sclerosis of the diaphyses and evidence of a modelling defect in the metaphyses. The spine, ribs, clavicles, and pelvis all show some degree of sclerosis and defective modelling, but are less severely involved. According to the definition by Gorlin, this disorder would best be classified as craniodiaphyseal dysplasia. Distinguishing features in these two patients as contrasted to previously described cases include a greater degree of hyperostosis and sclerosis than that described for other patients with craniodiaphyseal dysplasia, apparent dominant transmission, and significant metaphyseal involvement.

Adult↗
PubMed1981

Benign subdural collections of infancy.

Four asymptomatic infants with macrocrania, abnormal transillumination, and characteristic computed tomography scans are described. All had bilateral subdural collections, normal brain size, modest ventricular enlargement, and prominent cerebral sulci and interhemispheric fissures. Although these latter findings are often interpreted as atrophy, these infants had normal development and rapidly growing heads. No treatment beyond diagnostic subdural punctures was performed. After up to 13 months of follow-up, the size of the subdural collections was either stable or decreasing in all four infants.

Cephalometry↗
PubMed1977

Electrically elicited blink reflex in normal neonates.

The electrically elicited blink reflex was tested in 30 full-term neonates. Of the two distinctly separate responses, early component R1 and late component R2, R1 was recorded in all but three infants. Its latency, 12.1+/-1.0 msec (mean+/-SD), was significantly greater than that in adults (10.6+/-0.8 msec) despite a considerably shorter length of the reflex arc in infants. Unlike the response in adults, R2 was elicited in only 20 of 30 infants, mostly on the side ipsilateral to the stimulus. The reduction of reflex excitability of R2 in neonates must occur primarily at the level of interneurons since oligosynaptic R1 was elicited with ease whereas polysynaptic R2 was not.

Age Factors↗
PubMed1977

EEG in benign intracranial hypertension.

A retrospective study of standard EEG's obtained from 26 patients with benign intracranial hypertension was done with particular emphasis on the effect of age on the EEG findings. Abnormal EEG's were found in 10 patients, all were less than 20 years of age. Although no consistent pattern of EEG abnormality could be identified, there appears to be a difference between the immature and adult brain in the EEG response to intracranial hypertension.

Adolescent↗
PubMedDate not supplied

Dipole reversal: an ictal feature of benign partial epilepsy with centrotemporal spikes.

Benign epilepsy with centrotemporal spikes (BECTS) is characterized by brief stereotypical partial seizures with motor and/or sensory symptoms with frequent secondarily generalized seizures. The interictal EEG shows slow, disphasic, high-voltage spikes in the centrotemporal areas. The few published examples of ictal tracings depict focal rhythmic sharp waves and spikes without significant postictal slowing. We report an ictal event in BECTS that is unusual in the evolution and polarity of the ictal discharges. In this subclinical seizure, ictal multiple spike and wave discharges appear as a dipole: they are electropositive in T3-C3 and negative in F3. These surface positive epileptic discharges are unique and require explanation. We postulate that the seizure discharge arises in the depths of the sylvian fissure involving folded cortical areas. This occurs in such a way that the negative component of the discharges is concealed from the scalp electrodes. Thus, a relative positivity is recorded on the surface. This represents a dipole reversal relative to the interictal discharges (characteristic of BECTS). This geometrical explanation allows us to avoid postulating an unusual mechanism of generation of this seizure.

Child↗